Cavernous Transformation Research Articles

A Novel Preoperative Classification System for Selecting Suitable Surgeries in Liver Transplant Patients with Portal Vein Cavernous Transformation

Background To evaluate the new preoperative Changgung classification (CC) system of portal vein thrombosis (PVT) in choosing suitable operative procedures to reconstruct portal veins during liver transplantation (LT) in patients with portal vein cavernous transformation (PVCT). Methods This retrospective observational study analyzed data from allograft LTs performed for various liver diseases Results The study included 22 males and 4 females with LT indications comprising cirrhosis (n = 9), hepatocellular carcinoma (n = 12), PVCT (n = 2), liver failure from fulminant hepatitis B (n = 1), dysfunction of transplanted liver (n = 1), and chronic rejection of transplanted liver (n = 1). Patients were classified according to Yerdel (21 Yerdel II and 5 Yerdel III) and CC (C1–C5). In total 16 simple operations were performed on C1–C3 cases and 9 complex operations on C4–C5 cases, with one additional simple operation. The distribution according to the Yerdel classification was 16 simple and 5 complex operations in Yerdel II cases and 1 simple and 4 complex operations in Yerdel III cases. The median follow-up time was 27.5 months with overall one-year and three-year OS rates of 88.1% and 83.9% for the cohort. Specifically, the one-year OS rates for patients classified as C1-3 vs. C4-5 were 93.3% and 80.0%, while the three-year OS rates were 86.7% and 80.0%, respectively (p = 0.526). Conclusion The CC proposed in this study shows comparable potential to the Yerdel classification in preoperatively identifying the need for complex surgical techniques in LT patients with PVCT and may also have predictive power for the survival benefits following LT.

Debating and Expanding on the Midgut Volvulus Case: A New Perspective

Since midgut volvulus is a rare but serious condition featuring twisted intestines, this presents significant problems with diagnosis and treatment- especially for adults. Early diagnosis and swift action are important in preventing such complications as juxtaposition of the bowel loops with volvulus, necrosis, etc. This study is based on recent developments of a case documented in then Jordanian American Physicians Academy Journal, and makes clear the difficulties involved in diagnosing midgut volvulus in a patient with cavernous transformation of the portal vein. Entering oublic health and reaching out to each discipline, means T.The fact that this study involved adult and pediatric cases demonstrates the need for advanced imaging technology and individualized treatment plans in order to improve patient outcomes.

Transjugular mesenteric-caval shunt for portal vein cavernous transformation with recurrent variceal bleeding: preliminary results.

This study aimed to evaluate the feasibility, safety, and efficacy of the transjugular mesenteric-caval shunt (TMCS) as a treatment for the cavernous transformation of the portal vein (CTPV) and recurrent variceal bleeding. This retrospective case series was conducted with approval from the institutional review board. It involved seven patients diagnosed with CTPV and recurrent variceal bleeding who underwent the TMCS procedure. We analyzed the rate of procedural complications, incidents of rebleeding, stent stenosis, hepatic encephalopathy, and overall survival to assess treatment outcomes. The TMCS was successfully performed in all seven patients without any life-threatening complications. Postoperatively, one patient developed a lung infection and pleural effusion, which resolved with appropriate treatment. Additionally, two patients experienced an increase in total bilirubin levels, but there was no further deterioration in liver function. The median portal pressure gradient significantly decreased from a preoperative value of 27 mmHg (range 20-36 mmHg) to a postoperative value of 6 mmHg (range 4-11 mmHg). A notable improvement was observed in one cirrhotic patient, with liver function progressing from Child-Pugh class B (score 9) to class A (score 6). Over a median follow-up period of 14 months (range 7-18 months), none of the patients encountered rebleeding, stent stenosis, hepatic encephalopathy, or mortality. The TMCS appears to be a viable and effective alternative for managing CTPV with recurrent variceal bleeding. Its long-term outcome requires further evaluation. TMCS provides a promising treatment for patients with life-threatening CTPV complications when occluded portal vein cannot be recanalized and portal vein recanalization TIPS is not an option. Performing TIPS in patients with portal vein cavernoma is complex due to the requirement for recanalization of the occluded portal vein. Creating a mesenteric-caval shunt through a transjugular approach is a feasible technique. Establishing a TMCS provides a means to manage life-threatening complications arising from portal vein cavernoma.

Portal vein stenting blocked the inflow tract and completely resolved bile duct varices, formed by cavernous transformation of the portal vein.

There is no established treatment for bleeding bile duct varices (BDVs). We report the first case of portal vein (PV) stenting completely eradicating bleeding BDVs. A 70-year-old male with malignant lymphoma developed BDVs due to PV obstruction, which had caused compression and stricture of the distal bile duct. Endoscopic retrograde cholangiography was performed to evaluate the stricture and bleeding from the ruptured BDV was observed. Endoscopic hemostasis was difficult, requiring reopening of the extra-hepatic PV and reducing the blood flow to the BDVs for hemostasis. Therefore, PV stenting was performed. During the procedure, portal angiography confirmed an inflow tract to the BDVs. Therefore, covered stents were placed in the PV and adjusted to block the inflow tract to the BDVs at the distal end. After stenting, the BDVs were successfully blocked and all PV blood flowed through the stent placed in the extra-hepatic PV. Two weeks after stenting, the BDVs had disappeared completely and the bleeding has not recurred for months. We experienced a case in which PV stenting not only reopened an obstructed PV but also successfully occluded the inflow tract. This case demonstrates the potential of PV stenting for the treatment of hemorrhagic BDVs.

Beyond the Rex: an innovative meso-intrahepatic portal vein bypass for late-onset extrahepatic portal vein occlusion after living donor liver transplantation.

The Meso-Rex bypass (MRB) is recognized as an effective treatment for portal hypertension secondary to extrahepatic portal vein occlusion (EHPVO) both in the pediatric and adult population, within or outside the context of liver transplantation. It is the preferred surgical treatment in most centers because not only does it addresses the portal hypertension, but also restores physiologic portal hepatopetal flow. However, the Rex recess, the landmark for this technique, may not be safely accessible in some patients. We present a 22-year-old male who underwent living donor liver transplant (LDLT) for neonatal hepatitis. He presented with variceal bleeding due to EHPVO at 13 years after transplant. Various endoscopic, radiologic, and surgical interventions were employed to address the recurrent gastrointestinal bleeding, but results have been unsatisfactory. We performed a meso-intrahepatic portal vein bypass (MIPVB), an innovative alternative to the MRB, for this patient with extensive post-operative adhesions, perihilar collaterals, and cavernous transformation. MIPVB creation in patients where the Rex recess is inaccessible is technically challenging. But with a multidisciplinary team approach, meticulous preoperative planning, and close follow-up, the authors have demonstrated that it is a safe and feasible option for patients with late-onset EHPVO after liver transplantation.

Efficacy and safety of precision-guided transjugular extrahepatic portosystemic shunt (TEPS) in the management of cavernous transformation of the portal vein with portal hypertension: a case series

Background and aimsPerforming a Transjugular intrahepatic portal system shunt (TIPS) in patients with portal vein cavernous transformation (CTPV) poses significant challenges. As an alternative, transjugular extrahepatic portal vein shunt (TEPS) may offer a potential solution for these patients. Nonetheless, the effectiveness and safety of TEPS remain uncertain. This case series study aimed to evaluate the efficacy and safety of TEPS in treating patients with CTPV portal hypertension complications.MethodsThe study encompassed a cohort of 22 patients diagnosed with CTPV who underwent TEPS procedures. Of these, 13 patients manifested recurrent hemorrhagic episodes subsequent to conventional therapies, 8 patients grappled with recurrent or refractory ascites, and 1 patient experienced acute bleeding but refused endoscopic treatment. Comprehensive postoperative monitoring was conducted for all patients to rigorously evaluate both the technical and clinical efficacy of the intervention, as well as long-term outcomes.ResultsThe overall procedural success rate among the 22 patients was 95.5% (21/22).During the TEPS procedure, nine patients were guided by percutaneous splenic access, three patients were guided by percutaneous hepatic access, five patients were guided by transmesenteric vein access from the abdomen, and two patients were guided by catheter marking from the hepatic artery. Additionally, guidance for three patients was facilitated by pre-existing TIPS stents. The postoperative portal pressure gradient following TEPS demonstrated a statistically significant decrease compared to preoperative values (24.95 ± 3.19 mmHg vs. 11.48 ± 1.74 mmHg, p < 0.01).Although three patients encountered perioperative complications, their conditions ameliorated following symptomatic treatment, and no procedure-related fatalities occurred. During a median follow-up period of 14 months, spanning a range of 5 to 39 months, we observed four fatalities. Specifically, one death was attributed to hepatocellular carcinoma, while the remaining three were ascribed to chronic liver failure. During the follow-up period, no instances of shunt dysfunction were observed.ConclusionsPrecision-guided TEPS appears to be a safe and efficacious intervention for the management of CTPV.

Successful management of VTE with essential thrombocythemia and cavernous transformation of the portal vein in early pregnancy: a case report

Due to the thrombohemorrhagic potential of essential thrombocythemia, pregnancy complicated by essential thrombocythemia should be recognized as a risk factor for obstetric complications. Here, we report the case of a patient with essential thrombocythemia with two significantly different pregnancy outcomes. Her first pregnancy (at 30 years of age) ended with an uneventful term delivery. However, the patient progressed to cavernous transformation of the portal vein in the period between her two pregnancies and subsequently experienced deep venous thrombosis during the first trimester of her second pregnancy (at 36 years of age). The patient’s platelet count during pregnancy was within the normal range, so she ignored previous instances of essential thrombocytosis (at 26 years of age). The patient’s main symptom was unrelieved pain in her leg. After that, she was successfully treated with anticoagulant throughout her entire pregnancy, resulting in a term vaginal delivery. This case highlights the importance of assessing pregnant patients with essential thrombocythemia according to their risk stratification. Specifically, risk assessments for potential pregnancy complications should take into account advanced maternal age and a previous history of thrombosis. Patients with essential thrombocythemia should be encouraged to participate in preconception counseling for risk assessment and to initiate prophylactic anticoagulation as soon as possible.

Noncirrhotic Portal Hypertension – A Case Report on a Sequela of Portal Vein Cavernoma

Introduction: Increased pressures in the portal vein (portal hypertension) which occurs following portal vein thrombosis, results in cavernous transformation of the portal vein. Though portal vein thrombosis (PVT) is a frequent complication in cirrhotic patients, it may also exist as a basic vascular condition without any liver damage. Among the predisposing factors for portal vein cavernoma are deficiencies in protein C, S &amp; antithrombin III, antiphospholipid syndrome and mutations in factor V Leiden and JAK2. Determination of the aetiology aids in the management plan to not only relieve symptoms of the patient but also to treat the underlying cause. Gastroesophageal variceal bleeding, splenomegaly, portosystemic collaterals, and ultimately hematologic abnormalities are among the prominent clinical features. Case Presentation: We present a case of a 16-year-old male with portal vein cavernoma complicated by bleeding oesophageal varices presenting with a second episode of hematemesis and melena within a 10-year period. He underwent endoscopic variceal band ligation and was put on oral warfarin and propranolol. The patient was followed up once at the outpatient clinic after discharge without the laboratory investigations we requested due to financial constraints. He has since been lost to follow up. Conclusion: Bleeding oesophageal varices from noncirrhotic causes are common and a high index of suspicion is needed to make a diagnosis. Though investigations tailored towards identifying the underlying cause presents a challenge in a resource constrained setting like ours, management of complications and symptoms to reduce morbidity and mortality cannot be over-emphasized.

Management of a child with portopulmonal hypertension due to cavernous transformation of the portal vein and sple- norenal anastomosis

A clinical case of the development of portopulmonary hypertension in a child after splenorenal anastomosis is presented. The surgical intervention was performed to correct the congenital malformation — cavernous transformation of the portal vein, which led to portal hypertension and varicose veins of the esophagus and stomach. In the long-term postoperative period, pulmonary arterial hypertension, hypersplenism with bilinear cytopenia, and moderate liver failure developed. Combined therapy provided a temporary improvement of the patient status, therefore, during subsequent surgery, the size of the anastomosis was changed. This led to a sustained decrease in pulmonary artery blood pressure and clinical improvement in the boy’s condition.

Hepatic Hydatic Cyst Causing Portal Hypertension and Cavernous Transformation in a Pediatric Patient.

Hepatic Hydatic Cyst Causing Portal Hypertension and Cavernous Transformation in a Pediatric Patient.

221 - Cavernous transformation of the portal vein, an unexpected source of anemia

221 - Cavernous transformation of the portal vein, an unexpected source of anemia

Portal vein stenting in recurrent or locally advanced peri-hilar cholangiocarcinoma

BackgroundRecurrent or locally advanced peri-hilar cholangiocarcinoma (PHCC) usually involves the portal vein (PV) leading to significant stenosis. With disease progression, clinical symptoms such as ascites, bleeding, and hepatic insufficiency are usually observed. Little is know about the benefit of PV stenting in relieving the symptoms associated to portal hypertension and allowing anticancer therapies. The aim of this study is to review our experience in PV stenting for PHCC patients. MethodsFrom 2014 to 2022, data from PHCC patients underwent PV stenting at Verona University Hospital, Italy, were reviewed. The indications were: gastrointestinal bleeding from esophagus-gastric varices, ascites not responsive to medical therapy, severe thrombocytopenia, liver insufficiency (hepatic jaundice, coagulopathy, and/or hyperammoniemia), or asymptomatic high-grade PV stenosis. Cavernous transformation and intrahepatic thrombosis in both sides of the liver were considered contraindication. Systematic anticoagulation therapy was not administered. ResultsTechnical success was achieved in all 16 (100 %) patients. The improvement of clinical symptoms were observed in 12 (75 %) patients. Anticancer therapy was administrated in 11 (69 %) patients. 2 (13 %) complications were observed: 1 biliary injury and 1 recurrent cholangitis that required a percutaneous trans-hepatic biliary drainage placement. Stent occlusion for tumor progression occurred in 1 patient and a re-stenting procedure was successfully performed. No case of thrombotic stent occlusion was observed during follow up. The 1-year stent patency was 86 % and the median patency period was 8 months (IQR, 4–12). ConclusionPV stenting is a feasible and safe palliative treatment that improves clinical condition, allow anticancer therapies, and provide a better quality of life.

Experience of adult liver transplantation with the severe portal vein cavernous transformation

Experience of adult liver transplantation with the severe portal vein cavernous transformation

Lieno-Adrenal Shunt a Rescue for Porto-Systemic Decompression in 23-Year-Old Female

Extra hepatic portal vein obstruction is one of the commonest cause of portal hypertension and upper gastrointestinal bleed in the third world. Portal vein cavernous transformation is one of the hallmarks of EHPVO. Despite various lesser invasive interventional approaches for management of EHPVO, surgical shunts still play an important role in long term treatment of patients with EHPVO. Here we present a case of 23 years old female who presented to the OPD with complains of dull aching abdominal pain for a year which was insidious on onset and gradually progressive. The pain was non-radiating, localized to the left upper quadrant of the abdomen which aggravated on lying on the left side. She underwent lieno-adrenal shunt for porto-systemic decompression and management of EHPVO. This case aims to highlight the use of leino-adrenal shunt as a feasible alternative to Linton’s shunt.

Successful management of portal hypertension with splenomegaly and cavernous transformation of the portal vein: a rare case report

Abstract Portal hypertension, often stemming from liver cirrhosis or vascular anomalies, can result in cavernous transformation of the portal vein, a rare condition associated with biliary obstruction, variceal hemorrhage, and splenomegaly. This case report details a unique occurrence of portal hypertension, splenomegaly, and cavernous transformation of the portal vein successfully managed through splenectomy and spleno-renal shunt. A 30-year-old female with a history of portal hypertension, portal gastropathy, and splenomegaly presented with left upper quadrant abdominal pain. She had previously undergone esophageal variceal ligation and required intermittent blood transfusions. Additional complications included easy bruising, heavy menstrual bleeding, and a prior episode of hematemesis. Clinical assessment confirmed splenomegaly, while a CT scan confirmed the diagnosis. A tailored surgical approach was chosen, leading to splenectomy and spleno-renal shunt.

A CLINICAL CASE OF PORTAL BILIOPATHY

Background. The term «portal biliopathy» (PB) refers to abnormalities of the entire biliary tract including intrahepatic and extrahepatic bile ducts, the cystic duct as well as the gallbladder in patients with portal hypertension. Objective. To describe a clinical case of portal biliopathy. Material and methods. Clinical observation of a patient aged 42, who was examined and treated at the 2nd City Clinical Hospital, Minsk, Belarus in February 2023. Results. Esophagogastroduodenoscopy and ultrasonography revealed portal hypertension, grade 2-3 esophageal varices and changes in the bile ducts. The subsequent use of computed tomography, magnetic resonance imaging, endoscopic retrograde cholangiopancreatography made it possible to identify cavernous transformation of portal vein due to occlusive thrombosis as well as portal biliopathy with extensive stenosis of the ductus choledochus. Conclusions. The presented clinical case demonstrates the asymptomatic course of a severe pathology known as extrahepatic portal vein obstruction (EHPVO) with the development of portal hypertension and portal biliopathy.

The Portal Vein: A Comprehensive Review.

Radiologists are familiar with the appearances of a normal portal vein; variations in its anatomy are commonplace and require careful consideration due to the implications for surgery. These alterations in portal vein anatomy have characteristic appearances that are clearly depicted on CT, MR, and US images. Similarly, there are numerous congenital and acquired disorders of the portal vein that are deleterious to its function and can be diagnosed by using imaging alone. Some of these conditions have subtle imaging features, and some are conspicuous at imaging but poorly understood or underrecognized. The authors examine imaging appearances of the portal vein, first by outlining the classic and variant anatomy and then by describing each of the disorders that impact portal vein function. The imaging appearances of portal vein abnormalities discussed in this review include (a) occlusion from and differentiation between bland thrombus and tumor in vein and the changes associated with resultant hepatic artery buffer response changes, cavernous transformation of the portal vein, and portal biliopathy; (b) ascending thrombophlebitis of the portal vein (pylephlebitis); (c) portal hypertension and its causes and sequelae; (d) the newly described disease entity portosinusoidal vascular disorder; and (e) intra- and extrahepatic shunts of the portal system, both congenital and acquired (including Abernethy malformations), and the associated risks. Current understanding of the pathophysiologic processes of each of these disorders is considered to aid the approach to reporting. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

S3570 Beckwith-Wiedemann Syndrome: A Rare Presentation of Chronic Portal Vein Thrombosis with Cavernous Transformation Extending to the Superior Mesenteric Vein

S3570 Beckwith-Wiedemann Syndrome: A Rare Presentation of Chronic Portal Vein Thrombosis with Cavernous Transformation Extending to the Superior Mesenteric Vein

Location and extent of cavernous transformation of the portal vein dictates different visceral side revascularization in Meso-Rex bypass

BackgroundAs an emerging standard of care for portal vein cavernous transformation (PVCT), Meso-Rex bypass (MRB) has been complicated and variated. The study aim was to propose a new classification of PVCT to guide MRB operations.MethodsDemographic data, the extent of extrahepatic PVCT, surgical methods for visceral side revascularization, intraoperative blood loss, operating time, changes in visceral venous pressure before and after MRB, postoperative complications and the condition of bypass vessels after MRB were extracted retrospectively from the medical records of 19 patients.ResultsThe median age of the patients (13 males and 6 females) was 32.5 years, while two patients were underage. Causes of PVCT can be summarized as follows: thrombophilia such as dysfunction of antithrombin III or proteins C; secondary to abdominal surgeries; secondary to abdominal infection or traumatic intestinal obstruction, and unknown causes. Intraoperatively, the median operation time was 9.5 h (7–13 h), and the intraoperative blood loss was 300 mL (100-1,600 mL). Ten cases used autologous blood vessels while 10 used allogeneic blood vessels. The vascular anastomosis was divided into the following types according to the site and approach: Type (T) 1-PV pedicel type, T2-confluence type, T3-major visceral vascular type; and T4-collateral visceral vascular type. Furthermore, the visceral venous pressure before and after MRB dropped significantly from 36 cmH2O (28–44) to 24.5 cmH2O (15–31) (P < 0.01). Postoperatively, one patient had delayed wound healing, two developed biochemical pancreatic fistulae, one experienced lymphatic leakage, the former caused by heat damage of the pancreatic tissues, the latter by cutting lymphatic vessels in the mesentery or removing the local lymph nodes during the process of separating the superior mesenteric vein, and one was re-operated on for an intervening intestinal fistulae. Postoperative enhanced CT scans revealed a significant improvement in abdominal varix in the patients with patent bypass, and at the 1-year postoperative follow-up, enhanced CT scans of six patients showed that the long axis of the spleen was reduced by ≥ 2 cm.ConclusionsMRB can effectively reduce visceral venous pressure in patients with PVCT. It is feasible to determine the PVCT type according to the extent of involvement and to choose individualized visceral side revascularization performances.

Natural History and Outcomes of Cavernous Transformation of the Portal Vein in Cirrhosis.

Cavernous transformation of the portal vein can occur after portal vein thrombosis (PVT). In this study, we investigated clinical complications associated with cavernous transformation in the context of cirrhosis and PVT. In this retrospective cohort analysis, 204 patients with cirrhosis and PVT with or without cavernous transformation were identified using MUSC's Clinical Data Warehouse between January 1, 2013, through December 31, 2019. Complete demographic data, clinical history, and laboratory tests were abstracted from the electronic medical record. Of 204 patients, 41 (20%) had cavernous transformation. MELD, Child-Pugh, and Charlson Comorbidity Index scores were similar among groups. There were no significant differences in the prevalence of esophageal varices (with or without bleeding), splenomegaly, or hepatic encephalopathy in patients with and without cavernous transformation, although ascites tended to be lower in patients with cavernous transformation (31/41 (76%) vs 142/163 (87%), p = 0.06). Patients with cavernous transformation were significantly less likely to have hepatocellular carcinoma (HCC) (13/41 (32%) vs 81/163 (50%), p < 0.05) and had significantly lower APRI (1.4 vs 2.0, p < 0.05) and Fib-4 (4.7 vs 6.5, p < 0.05). Patients with cavernous transformation had lower 5-year mortality (12/41 (29%) vs 81/163 (49%) died, p = 0.06). The 10-year mortality of patients with cavernous transformation without HCC was significantly lower than in those without cavernous transformation (8/28 (29%) vs 46/82 (56%), respectively, p < 0.05). Patients with cavernous transformation appeared to have better outcomes than those without cavernous transformation.